SitesBLAST

4y1pB Crystal structure of 3-isopropylmalate dehydrogenase (saci_0600) from sulfolobus acidocaldarius complex with 3-isopropylmalate and mg2+ (see paper)
49% identity, 97% coverage: 4:322/328 of query aligns to 4:331/336 of 4y1pB

query
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4y1pB

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active site: Y123 (= Y119), K169 (= K166), D200 (= D199), D224 (= D223), D228 (= D227)
binding 3-isopropylmalic acid: A78 (= A74), R85 (= R81), R95 (= R91), R116 (= R112), Y123 (= Y119), D224 (= D223)

6m3sB Dimeric isocitrate dehydrogenase from xanthomonas campestris pv. Campestris 8004
51% identity, 96% coverage: 2:315/328 of query aligns to 6:327/338 of 6m3sB

query
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6m3sB

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active site: Y128 (= Y119), K177 (= K166), D210 (= D199), D234 (= D223)
binding isocitrate calcium complex: T75 (≠ G67), S83 (≠ D75), N85 (≠ I77), R89 (= R81), R99 (= R91), R121 (= R112), Y128 (= Y119), D234 (= D223), D238 (= D227)
binding nicotinamide-adenine-dinucleotide: P72 (vs. gap), L73 (= L65), T75 (≠ G67), N85 (≠ I77), H266 (= H255), G267 (= G256), S268 (= S257), A269 (= A258), D271 (= D260), I272 (= I261), N279 (= N268)

4yb4A Crystal structure of homoisocitrate dehydrogenase from thermus thermophilus in complex with homoisocitrate, magnesium ion (ii) and nadh
49% identity, 99% coverage: 3:327/328 of query aligns to 3:331/333 of 4yb4A

query
sites
4yb4A

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active site: Y124 (= Y119), K170 (= K166), D203 (= D199), D227 (= D223), D231 (= D227)
binding (1R,2S)-1-hydroxybutane-1,2,4-tricarboxylic acid: S71 (≠ E71), R84 (≠ V78), R87 (= R81), R97 (= R91), R117 (= R112), Y124 (= Y119), D227 (= D223)
binding 1,4-dihydronicotinamide adenine dinucleotide: I12 (= I12), A69 (= A69), T70 (≠ G70), S71 (≠ E71), I201 (≠ Y197), N204 (≠ A200), L240 (= L236), E256 (= E252), H259 (= H255), G260 (= G256), S261 (= S257), A262 (= A258), D264 (= D260), I265 (= I261), N272 (= N268), D312 (= D308)

3asjB Crystal structure of homoisocitrate dehydrogenase in complex with a designed inhibitor (see paper)
49% identity, 99% coverage: 3:327/328 of query aligns to 3:331/333 of 3asjB

query
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3asjB

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G

D
|
D

I

I

V

L

S

S

D
|
D

L

L

I

A

A

A

G

G

L

L

V

V

G

G

L

L

G

G

L

L

A

A

P

P

S

S

A

G

N

N

I

I

G

G

E

D

K

T

Y

T

A

A

I

V

F

F

E

E

P

P

V
|
V

H

H

G

G

S

S

A

A

P

P

D

D

I

I

A

A

G

G

K

K

G

G

I

I

A

A

N

N

P

P

S

T

A

A

M

A

I

I

L

L

S

S

S

A

T

A

L

M

M

M

L

L

R

D

H

Y

F

L

G

G

Y

E

I

K

S

E

E

A

A

A

E

K

R

R

I

V

E

E

K

K

A

A

L

V

E

D

K

L

T

V

I

L

A

E

E

R

G

G

K

P

T

R

T

T

P

P

D

D

L

L

G

G

N

D

L

A

K

T

T

T

D

E

E

A

M

F

V

T

E

E

E

A

I

V

K

E

N

A

I

L

E

K

active site: Y124 (= Y119), K170 (= K166), D203 (= D199), D227 (= D223), D231 (= D227)
binding (2Z)-3-[(carboxymethyl)sulfanyl]-2-hydroxyprop-2-enoic acid: R84 (≠ V78), R97 (= R91), R117 (= R112), Y124 (= Y119), D227 (= D223), D231 (= D227), V258 (= V254)

3asjA Crystal structure of homoisocitrate dehydrogenase in complex with a designed inhibitor (see paper)
49% identity, 99% coverage: 3:327/328 of query aligns to 3:331/333 of 3asjA

query
sites
3asjA

K

R

I

I

A

C

V

L

I

I

P

E

G

G

D

D

G

G

I

I

G

G

K

H

E

E

V

V

M

I

S

P

A

A

C

A

L

R

K

R

V

V

L

L

E

E

A

A

F

T

D

G

F

L

P

P

F

L

E

E

Y

F

V

V

F

E

A

A

E

E

A

A

G

G

D

W

E

E

T

T

K

F

E

E

K

R

T

R

G

G

E

T

A

S

L

V

P

P

D

E

E

E

T

T

L

V

Q

E

I

K

C

I

K

L

E

S

S

C

S

H

A

A

V

T

L

L

F

F

G

G

A

A

G

T

E

S

T

P

A

T

A

R

D

K

V

V

-

P

-

G

-

F

-

G

-

A

I

I

V

R

R

Y

L

L

R

R

L

R

E

R

L

L

E

D

T

L

F

Y

A

A

N

N

L

V

R
|
R

P

P

A

A

K

K

S

S

Y

-

K

R

G

P

V

V

K

P

C

G

L

S

F

R

D

P

N

G

V

V

D

D

I

L

V

V

V

I

V

V

R
|
R

E

E

N

N

T

T

E

E

C

G

L

L

Y
|
Y

K

-

G

-

L

V

E

E

F

Q

E

E

V

R

K

R

K

Y

-

L

G

D

V

V

T

A

E

I

G

A

I

D

R

A

I

V

I

I

T

S

E

K

K

K

A

A

S

S

E

E

R

R

I

I

A

G

R

R

Y

A

A

A

F

L

E

R

L

I

A

A

R

E

R

G

D

R

G

P

R

R

K

K

R

T

V

L

T

H

A

I

L

A

H

H

K
|
K

A

A

N

N

V

V

M

L

K

P

K

L

T

T

C

Q

G

G

L

L

F

F

R

L

D

D

V

T

C

V

R

K

R

E

V

V

A

A

R

K

D

D

Y

F

D

P

D

L

I

V

E

N

F

V

S

Q

D

D

Y

I

I

V

D
|
D

A

N

G

C

C

A

M

M

Y

Q

L

L

V

V

L

M

D

R

P

P

W

E

R

R

F

F

D

D

V

V

I

I

V

V

T

T

N

N

M

L

F

L

G

G

D
|
D

I

I

V

L

S

S

D
|
D

L

L

I

A

A

A

G

G

L

L

V

V

G

G

L

L

G

G

L

L

A

A

P

P

S

S

A

G

N

N

I

I

G

G

E

D

K

T

Y

T

A

A

I

V

F

F

E

E

P

P

V

V

H

H

G

G

S

S

A

A

P

P

D

D

I

I

A

A

G

G

K

K

G

G

I

I

A

A

N

N

P

P

S

T

A

A

M

A

I

I

L

L

S

S

S

A

T

A

L

M

M

M

L

L

R

D

H

Y

F

L

G

G

Y

E

I

K

S

E

E

A

A

A

E

K

R

R

I

V

E

E

K

K

A

A

L

V

E

D

K

L

T

V

I

L

A

E

E

R

G

G

K

P

T

R

T

T

P

P

D

D

L

L

G

G

N

D

L

A

K

T

T

T

D

E

E

A

M

F

V

T

E

E

E

A

I

V

K

E

N

A

I

L

E

K

active site: Y124 (= Y119), K170 (= K166), D203 (= D199), D227 (= D223), D231 (= D227)
binding 3-[(carboxymethyl)sulfanyl]-2-oxopropanoic acid: R97 (= R91), R117 (= R112), Y124 (= Y119), D227 (= D223), D231 (= D227)

Q72IW9 Isocitrate/homoisocitrate dehydrogenase; Homoisocitrate dehydrogenase; HICDH; EC 1.1.1.286 from Thermus thermophilus (strain ATCC BAA-163 / DSM 7039 / HB27) (see 4 papers)
49% identity, 99% coverage: 3:327/328 of query aligns to 4:332/334 of Q72IW9

query
sites
Q72IW9

K

R

I

I

A

C

V

L

I

I

P

E

G

G

D

D

G

G

I

I

G

G

K

H

E

E

V

V

M

I

S

P

A

A

C

A

L

R

K

R

V

V

L

L

E

E

A

A

F

T

D

G

F

L

P

P

F

L

E

E

Y

F

V

V

F

E

A

A

E

E

A

A

G

G

D

W

E

E

T

T

K

F

E

E

K

R

T

R

G

G

E

T

A

S

L

V

P

P

D

E

E

E

T

T

L

V

Q
x
E

I

K

C

I

K

L

E

S

S

C

S

H

A

A

V

T

L

L

F

F

G

G

A

A

A
|
A

G
x
T

E
x
S

T

P

A

T

A

R

D

K

V

V

-

P

-

G

-

F

-

G

-

A

I

I

V
x
R

R
x
Y

L

L

R
|
R

L

R

E

R

L

L

E

D

T

L

F

Y

A

A

N

N

L

V

R
|
R

P

P

A

A

K

K

S

S

Y

-

K

R

G

P

V

V

K

P

C

G

L

S

F

R

D

P

N

G

V

V

D

D

I

L

V

V

V

I

V

V

R
|
R

E

E

N

N

T

T

E

E

C

G

L

L

Y
|
Y

K

-

G

-

L

V

E

E

F

Q

E

E

V

R

K

R

K

Y

-

L

G

D

V
|
V

T

A

E

I

G

A

I

D

R

A

I

V

I

I

T

S

E

K

K

K

A

A

S

S

E

E

R

R

I

I

A

G

R

R

Y

A

A

A

F

L

E

R

L

I

A

A

R

E

R

G

D

R

G

P

R

R

K

K

R

T

V

L

T

H

A

I

L

A

H

H

K
|
K

A

A

N
|
N

V

V

M

L

K

P

K

L

T

T

C

Q

G

G

L

L

F

F

R

L

D

D

V

T

C

V

R

K

R

E

V

V

A

A

R

K

D

D

Y

F

D

P

D

L

I

V

E

N

F

V

S

Q

D

D

Y

I

I

V

D
|
D

A

N

G

C

C

A

M
|
M

Y

Q

L

L

V

V

L

M

D

R

P

P

W

E

R

R

F
|
F

D

D

V

V

I

I

V

V

T

T

N

N

M

L

F

L

G

G

D
|
D

I

I

V

L

S

S

D
|
D

L

L

I

A

A

A

G

G

L

L

V
|
V

G

G

L

L

G

G

L

L

A

A

P

P

S

S

A

G

N

N

I

I

G

G

E

D

K

T

Y

T

A

A

I

V

F

F

E

E

P

P

V

V

H

H

G
|
G

S
|
S

A
|
A

P
|
P

D
|
D

I

I

A

A

G

G

K

K

G

G

I

I

A

A

N
|
N

P

P

S

T

A

A

M

A

I

I

L

L

S

S

S

A

T

A

L

M

M

M

L

L

R

D

H

Y

F

L

G

G

Y

E

I

K

S

E

E

A

A

A

E

K

R

R

I

V

E

E

K

K

A

A

L

V

E

D

K

L

T

V

I

L

A

E

E

R

G

G

K

P

T
x
R

T

T

P

P

D

D

L

L

G

G

N

D

L

A

K

T

T

T

D

E

E

A

M

F

V

T

E

E

E

A

I

V

K

E

N

A

I

L

E

K

E57 (≠ Q56) mutation to V: Confers enzyme activity with 3-isopropylmalate; when associated with I-72; M-85; A-86; T-208; Y-217; M-238 and M-310.
ATS 70:72 (≠ AGE 69:71) binding NADH
S72 (≠ E71) binding in other chain; mutation to I: Confers enzyme activity with 3-isopropylmalate; when associated with V-57; M-85; A-86; T-208; Y-217; M-238 and M-310.
R85 (≠ V78) binding in other chain; mutation to M: Confers enzyme activity with 3-isopropylmalate; when associated with V-57; I-72; A-86; T-208; Y-217; M-238 and M-310.; mutation to V: Confers low enzyme activity with 3-isopropylmalate. Reduces activity with homoisocitrate. Abolishes activity with isocitrate.
Y86 (≠ R79) mutation to A: Confers enzyme activity with 3-isopropylmalate; when associated with V-57; I-72; M-85; T-208; Y-217; M-238 and M-310.
R88 (= R81) binding in other chain
R98 (= R91) binding in other chain
R118 (= R112) binding in other chain
Y125 (= Y119) binding in other chain; mutation to A: Reduces catalytic efficiency with isocitrate.
V135 (= V130) mutation to M: Formation of homodimers instead of homotetramers. Increased affinity for isocitrate. Reduces enzyme activity with isocitrate.
K171 (= K166) binding (2R,3S)-homoisocitrate
N173 (= N168) binding (2R,3S)-homoisocitrate; binding NADH
D204 (= D199) binding Mg(2+)
M208 (= M203) mutation to T: Confers enzyme activity with 3-isopropylmalate; when associated with V-57; I-72; M-85; A-86; T-208; Y-217; M-238 and M-310.
F217 (= F212) mutation to Y: Confers enzyme activity with 3-isopropylmalate; when associated with V-57; I-72; M-85; A-86; T-208; M-238 and M-310.
D228 (= D223) binding Mg(2+)
D232 (= D227) binding Mg(2+)
V238 (= V233) mutation to M: Confers enzyme activity with 3-isopropylmalate; when associated with V-57; I-72; M-85; A-86; T-208; Y-217; and M-310.
GSAPD 261:265 (= GSAPD 256:260) binding NADH
N273 (= N268) binding NADH
R310 (≠ T305) mutation to M: Confers enzyme activity with 3-isopropylmalate; when associated with V-57; I-72; M-85; A-86; T-208; Y-217; and M-238.

6kdyA Crystal structure of the alpha bata heterodimer of human idh3 in complex with NAD. (see paper)
44% identity, 100% coverage: 1:328/328 of query aligns to 2:335/335 of 6kdyA

query
sites
6kdyA

M

V

K

Q

K

T

I

V

A

T

V

L

I

I

P

P

G

G

D

D

G

G

I

I

G

G

K

P

E

E

V

I

M

S

S

A

A

A

C

V

L

M

K

K

V

I

L

F

E

D

A

A

F

A

D

K

F

A

P

P

F

I

E

Q

Y

W

-

E

-

R

-

N

V

V

F

T

A

A

E

I

A

Q

G

G

D

P

E

G

T

G

K

K

E

W

K

-

T

-

G

-

E

-

A

M

L

I

P

P

D

S

E

E

T

A

L

K

Q

E

I

S

C

M

K

D

E

K

S

N

S

K

A

M

V

G

L

L

F

K

G

G

-
x
P

-
x
L

-

K

-
x
T

-

P

-

I

A

A

G

G

E

H

T

P

A

S

A

M

D
x
N

V

L

I

L

V

L

R

R

L

K

R

T

L

F

E

D

L

L

E

-

T

-

F

Y

A

A

N

N

L

V

R

R

P

P

A

C

K

V

S

S

Y

I

K

E

G

G

V

Y

K

K

C

T

L

P

F

Y

D

T

N

D

V

V

D

N

I

I

V

V

V

T

V

I

R

R

E

E

N

N

T

T

E

E

C

G

L

E

Y
|
Y

K

S

G

G

L

I

E

E

F

H

E

V

V

I

K

V

K

D

G

G

V

V

T

V

E

Q

G

S

I

I

R

K

I

L

I

I

T

T

E

E

K

G

A

A

S

S

E

K

R

R

I

I

A

A

R

E

Y

F

A

A

F

F

E

E

L

Y

A

A

R

R

R

N

D

N

G

H

R

R

K

S

R

N

V

V

T

T

A

A

L

V

H

H

K
|
K

A

A

N

N

V

I

M

M

K

R

K

M

T

S

C

D

G

G

L

L

F

F

R

L

D

Q

V

K

C

C

R

R

R

E

V

V

A

A

R

E

D

S

Y

C

D

K

D

D

I

I

E

K

F

F

S

N

D

E

Y

M

Y

Y

I

L

D
|
D

A

T

G

V

C

C

M

L

Y

N

L

M

V

V

L

Q

D

D

P

P

W

S

R

Q

F

F

D

D

V

V

I

L

V

V

T

M

T

P

N

N

M

L

F

Y

G

G

D
|
D

I

I

V

L

S

S

D

D

L

L

I

C

A

A

G

G

L

L

V

I

G

G

L

L

G

G

L

V

A

T

P

P

S

S

A

G

N

N

I

I

G

G

E

A

K

N

-

G

Y

V

A

A

I

I

F

F

E

E

P

S

V

V

H
|
H

G
|
G

S
x
T

A
|
A

P

P

D
|
D

I
|
I

A

A

G

G

K

K

G

D

I

M

A

A

N
|
N

P

P

S

T

A

A

M

L

I

L

L

L

S

S

S

A

T

V

L

M

M

M

L

L

R

R

H

H

F

M

G

G

Y

L

I

F

S

D

E

H

A

A

E

A

R

R

I

I

E

E

K

A

A

A

L

C

E

F

K

A

T

T

I

I

A

K

E

D

G

G

K

K

T

S

-

L

T

T

P

K

D
|
D

L

L

G

G

N

N

L

A

K

K

T

C

D

S

E

D

M

F

V

T

E

E

I

I

I

C

K

R

N

R

I

V

E

K

E

D

active site: Y124 (= Y119), K171 (= K166), D204 (= D199), D228 (= D223)
binding nicotinamide-adenine-dinucleotide: P69 (vs. gap), L70 (vs. gap), T72 (vs. gap), N82 (≠ D75), H261 (= H255), G262 (= G256), T263 (≠ S257), A264 (= A258), D266 (= D260), I267 (= I261), N274 (= N268), D315 (= D308)

6kdeA Crystal structure of the alpha beta heterodimer of human idh3 in complex with ca(2+) (see paper)
44% identity, 100% coverage: 1:328/328 of query aligns to 2:335/336 of 6kdeA

query
sites
6kdeA

M

V

K

Q

K

T

I

V

A

T

V

L

I

I

P

P

G

G

D

D

G

G

I

I

G

G

K

P

E

E

V

I

M

S

S

A

A

A

C

V

L

M

K

K

V

I

L

F

E

D

A

A

F

A

D

K

F

A

P

P

F

I

E

Q

Y

W

-

E

-

R

-

N

V

V

F

T

A

A

E

I

A

Q

G

G

D

P

E

G

T

G

K

K

E

W

K

-

T

-

G

-

E

-

A

M

L

I

P

P

D

S

E

E

T

A

L

K

Q

E

I

S

C

M

K

D

E

K

S

N

S

K

A

M

V

G

L

L

F

K

G

G

-

P

-

L

-

K

-

T

-

P

-

I

A

A

G

G

E

H

T

P

A

S

A

M

D

N

V

L

I

L

V

L

R

R

L

K

R

T

L

F

E

D

L

L

E

-

T

-

F

Y

A

A

N

N

L

V

R

R

P

P

A

C

K

V

S

S

Y

I

K

E

G

G

V

Y

K

K

C

T

L

P

F

Y

D

T

N

D

V

V

D

N

I

I

V

V

V

T

V

I

R

R

E

E

N

N

T

T

E

E

C

G

L

E

Y
|
Y

K

S

G

G

L

I

E

E

F

H

E

V

V

I

K

V

K

D

G

G

V

V

T

V

E

Q

G

S

I

I

R

K

I

L

I

I

T

T

E

E

K

G

A

A

S

S

E

K

R

R

I

I

A

A

R

E

Y

F

A

A

F

F

E

E

L

Y

A

A

R

R

R

N

D

N

G

H

R

R

K

S

R

N

V

V

T

T

A

A

L

V

H

H

K
|
K

A

A

N

N

V

I

M

M

K

R

K

M

T

S

C

D

G

G

L

L

F

F

R

L

D

Q

V

K

C

C

R

R

R

E

V

V

A

A

R

E

D

S

Y

C

D

K

D

D

I

I

E

K

F

F

S

N

D

E

Y

M

Y

Y

I

L

D
|
D

A

T

G

V

C

C

M

L

Y

N

L

M

V

V

L

Q

D

D

P

P

W

S

R

Q

F

F

D

D

V

V

I

L

V

V

T

M

T

P

N

N

M

L

F

Y

G

G

D
|
D

I

I

V

L

S

S

D
|
D

L

L

I

C

A

A

G

G

L

L

V

I

G

G

L

L

G

G

L

V

A

T

P

P

S

S

A

G

N

N

I

I

G

G

E

A

K

N

-

G

Y

V

A

A

I

I

F

F

E

E

P

S

V

V

H

H

G

G

S

T

A

A

P

P

D

D

I

I

A

A

G

G

K

K

G

D

I

M

A

A

N

N

P

P

S

T

A

A

M

L

I

L

L

L

S

S

S

A

T

V

L

M

M

M

L

L

R

R

H

H

F

M

G

G

Y

L

I

F

S

D

E

H

A

A

E

A

R

R

I

I

E

E

K

A

A

A

L

C

E

F

K

A

T

T

I

I

A

K

E

D

G

G

K

K

T

S

-

L

T

T

P

K

D

D

L

L

G

G

N

N

L

A

K

K

T

C

D

S

E

D

M

F

V

T

E

E

I

I

I

C

K

R

N

R

I

V

E

K

E

D

active site: Y124 (= Y119), K171 (= K166), D204 (= D199), D228 (= D223)
binding calcium ion: D228 (= D223), D232 (= D227)

P50213 Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial; Isocitric dehydrogenase subunit alpha; NAD(+)-specific ICDH subunit alpha; EC 1.1.1.41 from Homo sapiens (Human) (see 5 papers)
44% identity, 100% coverage: 1:328/328 of query aligns to 31:364/366 of P50213

query
sites
P50213

M

V

K

Q

K

T

I

V

A

T

V

L

I

I

P

P

G

G

D

D

G

G

I

I

G

G

K

P

E

E

V

I

M

S

S

A

A

A

C

V

L

M

K

K

V

I

L

F

E

D

A

A

F

A

D

K

F

A

P

P

F

I

E

Q

Y

W

-

E

-

R

-

N

V

V

F

T

A

A

E

I

A

Q

G

G

D

P

E

G

T

G

K

K

E

W

K

-

T

-

G

-

E

-

A

M

L

I

P

P

D

S

E

E

T

A

L

K

Q

E

I

S

C

M

K

D

E

K

S

N

S

K

A

M

V

G

L

L

F

K

G

G

-

P

-

L

-

K

-

T

-

P

-

I

A

A

G

G

E

H

T

P

A

S

A

M

D

N

V

L

I

L

V

L

R
|
R

L

K

R

T

L

F

E

D

L

L

E

-

T

-

F

Y

A
|
A

N

N

L

V

R
|
R

P

P

A

C

K

V

S

S

Y

I

K

E

G

G

V

Y

K

K

C

T

L

P

F

Y

D

T

N

D

V

V

D

N

I

I

V

V

V

T

V

I

R
|
R

E

E

N

N

T

T

E

E

C

G

L
x
E

Y
|
Y

K

S

G

G

L

I

E

E

F

H

E

V

V

I

K

V

K

D

G

G

V

V

T

V

E

Q

G

S

I

I

R
x
K

I

L

I

I

T

T

E

E

K

G

A
|
A

S

S

E

K

R

R

I

I

A

A

R

E

Y

F

A

A

F

F

E

E

L

Y

A

A

R

R

R

N

D

N

G

H

R

R

K

S

R

N

V

V

T

T

A

A

L

V

H

H

K
|
K

A

A

N
|
N

V

I

M
|
M

K

R

K

M

T

S

C
x
D

G

G

L

L

F

F

R

L

D

Q

V

K

C

C

R

R

R

E

V

V

A

A

R

E

D

S

Y

C

D

K

D

D

I

I

E

K

F

F

S

N

D

E

Y

M

Y

Y

I

L

D
|
D

A

T

G

V

C

C

M

L

Y

N

L
x
M

V

V

L

Q

D

D

P

P

W

S

R

Q

F

F

D

D

V

V

I

L

V

V

T

M

T

P

N

N

M

L

F
x
Y

G

G

D
|
D

I

I

V

L

S

S

D
|
D

L

L

I

C

A

A

G

G

L

L

V

I

G

G

L

L

G

G

L

V

A

T

P

P

S

S

A

G

N

N

I

I

G

G

E

A

K

N

-

G

Y

V

A

A

I

I

F

F

E

E

P

S

V

V

H

H

G

G

S

T

A

A

P

P

D

D

I

I

A

A

G

G

K

K

G

D

I

M

A

A

N

N

P
|
P

S

T

A

A

M

L

I

L

L

L

S

S

S

A

T

V

L
x
M

M

M

L

L

R
|
R

H

H

F

M

G

G

Y

L

I

F

S

D

E

H

A

A

E

A

R

R

I

I

E

E

K

A

A

A

L

C

E

F

K

A

T

T

I

I

A

K

E

D

G

G

K

K

T

S

-

L

T

T

P

K

D

D

L

L

G

G

N

N

L

A

K

K

T

C

D

S

E

D

M

F

V

T

E

E

I

I

I

C

K

R

N

R

I

V

E

K

E

D

R115 (= R79) binding substrate
A122 (= A88) to T: in RP90; uncertain significance; dbSNP:rs756333430
R125 (= R91) binding substrate
R146 (= R112) binding substrate
E152 (≠ L118) mutation to A: No significant effect on the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate and ADP.
Y153 (= Y119) Critical for catalysis; mutation to F: Complete loss of activity of the heterotetramer, heterodimer composed of IDH3A and IDH3B subunits and the heterodimer composed of IDH3A and IDH3G subunits with no effect on their oligomeric states.
K169 (≠ R135) mutation to A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate and ADP.
A175 (= A141) to V: in RP90; uncertain significance; dbSNP:rs765473830
K200 (= K166) Critical for catalysis; mutation to A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate.
N202 (= N168) mutation to A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate.
M204 (= M170) to I: in RP90; uncertain significance
D208 (≠ C174) mutation to A: Complete loss of the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate and ADP.
D233 (= D199) binding Mg(2+)
M239 (≠ L205) to T: in RP90; uncertain significance; dbSNP:rs2074707744
Y255 (≠ F221) mutation to A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate and ADP.
D257 (= D223) binding Mg(2+)
D261 (= D227) binding Mg(2+)
P304 (= P269) to H: in RP90; uncertain significance; dbSNP:rs756712426
M313 (≠ L278) to T: in RP90; uncertain significance; dbSNP:rs149862950
R316 (= R281) to C: in RP90; uncertain significance; dbSNP:rs770798851

6l59A Crystal structure of the alpha gamma heterodimer of human idh3 in complex with cit, mg and atp binding at allosteric site and mg, atp binding at active site. (see paper)
44% identity, 100% coverage: 1:328/328 of query aligns to 1:323/325 of 6l59A

query
sites
6l59A

M

V

K

Q

K

T

I

V

A

T

V

L

I

I

P

P

G

G

D

D

G

G

I
|
I

G

G

K

P

E

E

V

I

M

S

S

A

A

A

C

V

L

M

K

K

V

I

L

F

E

D

A

A

F

A

D

K

F

A

P

P

F

I

E

Q

Y

W

V

-

F

-

A

-

E

-

A

-

G

-

D

-

E

E

T

E

K

R

E

N

K

V

T

T

G

A

-

I

-

Q

E

W

A

M

L

I

P

P

D

S

E

E

T

A

L

K

Q

E

I

S

C

M

K

D

E

K

S

N

S

K

A

M

V

G

L

L

F

K

G

G

A

P

A

L

G

K

E

T

T

P

A

S

A

M

D

N

V

L

I

L

V

L

R

R

L

K

R

T

L

F

E

D

L

L

E

-

T

-

F

Y

A

A

N

N

L

V

R

R

P

P

A

C

K

V

S

S

Y

I

K

E

G

G

V

Y

K

K

C

T

L

P

F

Y

D

T

N

D

V

V

D

N

I

I

V

V

V

T

V

I

R

R

E

E

N

N

T

T

E

E

C

G

L

E

Y
|
Y

K

S

G

G

L

I

E

E

F

H

E

V

V

I

K

V

K

D

G

G

V

V

T

V

E

Q

G

S

I

I

R

K

I

L

I

I

T

T

E

E

K

G

A

A

S

S

E

K

R

R

I

I

A

A

R

E

Y

F

A

A

F

F

E

E

L

Y

A

A

R

R

R

N

D

N

G

H

R

R

K

S

R

N

V

V

T

T

A

A

L

V

H

H

K
|
K

A

A

N

N

V

I

M

M

K

R

K

M

T

S

C

D

G

G

L

L

F

F

R

L

D

Q

V

K

C

C

R

R

R

E

V

V

A

A

R

E

D

S

Y

C

D

K

D

D

I

I

E

K

F

F

S

N

D

E

Y

M

Y

Y

I

L

D
|
D

A

T

G

V

C

C

M

L

Y

N

L

M

V

V

L

Q

D

D

P

P

W

S

R

Q

F

F

D

D

V

V

I

L

V

V

T

M

T

P

N

N

M

L

F

Y

G

G

D
|
D

I

I

V

L

S

S

D
|
D

L

L

I

C

A

A

G

G

L

L

V

I

G

G

L

L

G

G

L

V

A

T

P

P

S

S

A

G

N

N

I

I

G

G

E

A

K

N

-

G

Y

V

A

A

I

I

F

F

E

E

P

S

V

V

H
|
H

G
|
G

S
x
T

A
|
A

P

P

D

D

I

I

A

A

G

G

K

K

G

D

I

M

A

A

N
|
N

P

P

S

T

A

A

M

L

I

L

L

L

S

S

S

A

T

V

L

M

M

M

L

L

R

R

H

H

F

M

G

G

Y

L

I

F

S

D

E

H

A

A

E

A

R

R

I

I

E

E

K

A

A

A

L

C

E

F

K

A

T

T

I

I

A

K

E

D

G

G

K

K

T

S

-

L

T

T

P

K

D
|
D

L

L

G

G

N

N

L

A

K

K

T

C

D

S

E

D

M

F

V

T

E

E

I

I

I

C

K

R

N

R

I

V

E

K

E

D

active site: Y112 (= Y119), K159 (= K166), D192 (= D199), D216 (= D223)
binding adenosine-5'-triphosphate: I12 (= I12), H249 (= H255), G250 (= G256), T251 (≠ S257), A252 (= A258), N262 (= N268), D303 (= D308)
binding magnesium ion: D216 (= D223), D220 (= D227)

8grdA Crystal structure of a constitutively active mutant of the alpha beta heterodimer of human idh3 in complex with adp and mg (see paper)
44% identity, 100% coverage: 1:328/328 of query aligns to 1:325/325 of 8grdA

query
sites
8grdA

M

V

K

Q

K

T

I

V

A

T

V

L

I

I

P

P

G

G

D

D

G

G

I

I

G

G

K

P

E

E

V

I

M

S

S

A

A

A

C

V

L

M

K

K

V

I

L

F

E

D

A

A

F

A

D

K

F

A

P

P

F

I

E

Q

Y

W

V

-

F

-

A

-

E

-

A

-

G

-

D

-

E

E

T

E

K

R

E

N

K

V

T

T

-

A

-

I

-

Q

G

G

E

W

A

M

L

I

P

P

D

S

E

E

T

A

L

K

Q

E

I

S

C

M

K

D

E

K

S

N

S

K

A

M

V

G

L

L

F

K

G

G

A

P

A

L

G

-

E

K

T

T

A

P

D

S

V

M

I

N

V

L

R

L

L

L

R

R

L

K

E

T

L

F

E

D

T

L

F

Y

A

A

N

N

L

V

R

R

P

P

A

C

K

V

S

S

Y

I

K

E

G

G

V

Y

K

K

C

T

L

P

F

Y

D

T

N

D

V

V

D

N

I

I

V

V

V

T

V

I

R

R

E

E

N

N

T

T

E

E

C

G

L

E

Y

Y

K

S

G

G

L

I

E

E

F

H

E

V

V

I

K

V

K

D

G

G

V

V

T

V

E

A

G

S

I

I

R

K

I

L

I

I

T

T

E

E

K

G

A

A

S

S

E

K

R

R

I

I

A

A

R

E

Y

F

A

A

F

F

E

E

L

Y

A

A

R

R

R

N

D

N

G

H

R

R

K

S

R

N

V

V

T

T

A

A

L

V

H

H

K

K

A

A

N

N

V

I

M

M

K

R

K

M

T

S

C

D

G

G

L

L

F

F

R

L

D

Q

V

K

C

C

R

R

R

E

V

V

A

A

R

E

D

S

Y

C

D

K

D

D

I

I

E

K

F

F

S

N

D

E

Y

M

Y
|
Y

I

L

D

D

A
x
T

G

V

C

C

M

L

Y

N

L

M

V

V

L

Q

D

D

P

P

W

S

R

Q

F

F

D

D

V

V

I

L

V

V

T

M

T

P

N

N

M

L

F

Y

G

G

D
|
D

I

I

V

L

S

S

D
|
D

L

L

I

C

A

A

G

G

L

L

V

I

G

G

L

L

G

G

L

V

A

T

P

P

S

S

A

G

N

N

I

I

G

G

E

A

K

N

-

G

Y

V

A

A

I

I

F

F

E

E

P

S

V

V

H

H

G

G

S

T

A

A

P

P

D

D

I

I

A

A

G

G

K

K

G

D

I

M

A

A

N

N

P

P

S

T

A

A

M

L

I

L

L

L

S

S

S

A

T

V

L

M

M

M

L

L

R

R

H

H

F

M

G

G

Y

L

I

F

S

D

E

H

A

A

E

A

R

R

I

I

E

E

K

A

A

A

L

C

E

F

K

A

T

T

I

I

A

K

E

D

G

G

K

K

T

S

-

L

T

T

P

K

D

D

L

L

G

G

N

N

L

A

K

K

T

C

D

S

E

D

M

F

V

T

E

E

I

I

I

C

K

R

N

R

I

V

E

K

E

D

binding adenosine-5'-diphosphate: Y192 (= Y197), T195 (≠ A200)
binding magnesium ion: D218 (= D223), D222 (= D227)

Q9D6R2 Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial; Isocitric dehydrogenase subunit alpha; NAD(+)-specific ICDH subunit alpha; EC 1.1.1.41 from Mus musculus (Mouse) (see paper)
44% identity, 100% coverage: 1:328/328 of query aligns to 31:364/366 of Q9D6R2

query
sites
Q9D6R2

M

V

K

Q

K

T

I

V

A

T

V

L

I

I

P

P

G

G

D

D

G

G

I

I

G

G

K

P

E

E

V

I

M

S

S

A

A

S

C

V

L

M

K

K

V

I

L

F

E

D

A

A

F

A

D

K

F

A

P

P

F

I

E

Q

Y

W

-

E

-

R

-

N

V

V

F

T

A

A

E

I

A

Q

G

G

D

P

E

G

T

G

K

K

E

W

K

-

T

-

G

-

E

-

A

M

L

I

P

P

D

P

E

E

T

A

L

K

Q

E

I

S

C

M

K

D

E

K

S

N

S

K

A

M

V

G

L

L

F

K

G

G

-

P

-

L

-

K

-

T

-

P

-

I

A

A

G

G

E

H

T

P

A

S

A

M

D

N

V

L

I

L

V

L

R

R

L

K

R

T

L

F

E

D

L

L

E

-

T

-

F

Y

A

A

N

N

L

V

R

R

P

P

A

C

K

V

S

S

Y

I

K

E

G

G

V

Y

K

K

C

T

L

P

F

Y

D

T

N

D

V

V

D

N

I

I

V

V

V

T

V

I

R

R

E

E

N

N

T

T

E

E

C

G

L

E

Y

Y

K

S

G

G

L

I

E

E

F

H

E

V

V

I

K

V

K

D

G

G

V

V

T

V

E

Q

G

S

I

I

R

K

I

L

I

I

T

T

E

E

K

E

A

A

S

S

E

K

R

R

I

I

A

A

R

E

Y

F

A

A

F

F

E

E

L

Y

A

A

R

R

R

N

D

N

G

H

R

R

K

S

R

N

V

V

T

T

A

A

L

V

H

H

K

K

A

A

N

N

V

I

M

M

K

R

K

M

T

S

C

D

G

G

L

L

F

F

R

L

D

Q

V

K

C

C

R

R

R

E

V

V

A

A

R

E

D

N

Y

C

D

K

D

D

I

I

E

K

F

F

S

N

D
x
E

Y

M

Y

Y

I

L

D

D

A

T

G

V

C

C

M

L

Y

N

L

M

V

V

L

Q

D

D

P

P

W

S

R

Q

F

F

D

D

V

V

I

L

V

V

T

M

T

P

N

N

M

L

F

Y

G

G

D

D

I

I

V

L

S

S

D

D

L

L

I

C

A

A

G

G

L

L

V

I

G

G

L

L

G

G

L

V

A

T

P

P

S

S

A

G

N

N

I

I

G

G

E

A

K

N

-

G

Y

V

A

A

I

I

F

F

E

E

P

S

V

V

H

H

G

G

S

T

A

A

P

P

D

D

I

I

A

A

G

G

K

K

G

D

I

M

A

A

N

N

P

P

S

T

A

A

M

L

I

L

L

L

S

S

S

A

T

V

L

M

M

M

L

L

R

R

H

H

F

M

G

G

Y

L

I

F

S

D

E

H

A

A

E

A

R

K

I

I

E

E

K

A

A

A

L

C

E

F

K

A

T

T

I

I

A

K

E

D

G

G

K

K

T

S

-

L

T

T

P

K

D

D

L

L

G

G

N

N

L

A

K

K

T

C

D

S

E

D

M

F

V

T

E

E

I

I

I

C

K

R

N

R

I

V

E

K

E

D

E229 (≠ D195) mutation to K: Homozygous mutant mice exhibit retinal degeneration.

5greA Crystal structure of the alpha gamma heterodimer of human idh3 in complex with mg(2+), citrate and adp (see paper)
44% identity, 100% coverage: 1:328/328 of query aligns to 1:324/325 of 5greA

query
sites
5greA

M

V

K

Q

K

T

I

V

A

T

V

L

I

I

P

P

G

G

D

D

G

G

I

I

G

G

K

P

E

E

V

I

M

S

S

A

A

A

C

V

L

M

K

K

V

I

L

F

E

D

A

A

F

A

D

K

F

A

P

P

F

I

E

Q

Y

W

V

-

F

-

A

-

E

-

A

-

G

-

D

-

E

E

T

E

K

R

E

N

K

V

T

T

G

A

-

I

-

Q

E

W

A

M

L

I

P

P

D

S

E

E

T

A

L

K

Q

E

I

S

C

M

K

D

E

K

S

N

S

K

A

M

V

G

L

L

F

K

G

G

A

P

A

L

G

-